| First Author | Ohara S | Year | 2004 |
| Journal | Brain Res | Volume | 1022 |
| Issue | 1-2 | Pages | 244-6 |
| PubMed ID | 15353235 | Mgi Jnum | J:92532 |
| Mgi Id | MGI:3053472 | Doi | 10.1016/j.brainres.2004.06.037 |
| Citation | Ohara S, et al. (2004) Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus in a mouse model of Niemann-Pick disease type C. Brain Res 1022(1-2):244-6 |
| abstractText | Niemann-Pick disease type C (NP-C) is a progressive neurological disorder of lipid metabolism. The Balb/C npc1 mutant strain is a genetically authentic murine model of NPC, which reproduce the clinical and histologic features of human NP-C. In the present study, we show that cholecystokinin (CCK)-immunoreactive fibers in the thalamic VPL nuclei, which are densely distributed in controls, degenerate in NPC mice. This degeneration is associated with the appearance of CCK-immunoreactive axonal spheroids containing characteristic intracellular inclusions of NP-C. These observations provide supportive evidence of the occurrence of dying-back axonopathy of neurons in the dorsal column nuclei in this mouse model. |
